primary cardiac rhabdomyosarcoma
Introduction
Introduction to primary cardiac rhabdomyosarcoma Primary rhabdomyosarcomaofheart is a cardiac malignant tumor that is best developed from myocardial tissue except for angiosarcoma. Cardiac rhabdomyosarcoma is considered to be abnormal in development and may be differentiated from primitive mesenchymal cells. It is congenital. From a pathological point of view, rhabdomyosarcoma is a common and malignant mesenchymal tumor. The limbs, mostly located in or adjacent to the muscle, are nodular or lobulated, and the cut surface is fish-like, grayish or gray-red, often showing bleeding and necrosis. Microscopically, the tumor is mainly composed of various stages of differentiation. The composition of striated muscle cells. basic knowledge The proportion of illness: 0.0005% Susceptible population: the age of onset is more than 30 to 40 years old Mode of infection: non-infectious Complications: congestive heart failure, pericardial effusion, syncope, arrhythmia
Cause
Causes of primary cardiac rhabdomyosarcoma
Related to trauma (30%):
Tumor is a multi-cause, multi-stage and multiple-mutation disease, caused by alternating internal and external factors, the result of interaction, the cause of sarcoma is also the same. Soft tissue sarcoma has been proposed to be related to trauma, but there is no sufficient basis, others such as heredity The relationship between viruses, radiation, and flaky foreign bodies and sarcomas is mostly reported in individual cases or in animal experiments. The cause of sarcoma has not been determined so far and remains to be further explored.
Developmental abnormalities (30%):
Cardiac rhabdomyosarcoma is considered to be abnormal in development and may be differentiated from primitive mesenchymal cells. It is congenital. From a pathological point of view, rhabdomyosarcoma is a common and malignant mesenchymal tumor. The limbs, mostly located in or adjacent to the muscle, are nodular or lobulated, and the cut surface is fish-like, grayish or gray-red, often showing bleeding and necrosis. Microscopically, the tumor is mainly composed of various stages of differentiation. The composition of striated muscle cells, in adults, tumor cells more common pleomorphism, mainly spindle cells, with round, polygonal, scorpion or banded cells, tumor cytoplasm red staining, with longitudinal muscles The fibrils can be seen with horizontal stripes. In adolescents, the common rhabdomyosarcoma is mainly composed of small round cells with poor differentiation and cytoplasm red staining, often in the form of alveolar, while rhabdomyosarcoma in children is common in the eyelids and neck. It can also be found in areas without striated muscle tissue, such as bladder, vagina, etc. The tumor cells are mainly undifferentiated spindle-shaped cells and small round cells, and the horizontal stripes are difficult to see. Under electron microscope, the tumor cells of rhabdomyosarcoma Myofibrils visible under an optical microscope with a significant often difficult to find stripes.
Pathology (30%):
Primary cardiac rhabdomyosarcoma is divided into three types: polymorphic, acinar and embryonic.
(1) polymorphic rhabdomyosarcoma: large volume, sometimes with false capsule, soft, cut surface is fishy gray or red, often accompanied by hemorrhage or necrosis, rapid tumor growth, infiltration and destruction of surrounding tissue, tumor cells The shape is diverse, the cytoplasm is rich, the nucleus is irregular, the staining is deep, and the division is more.
(2) acinar rhabdomyosarcoma: the cut surface is grayish white, the texture is like rubber, the cytoplasm of tumor cells is less, eosinophilic, the tumor cells are arranged in a glandular shape, and there are also diffuse or nested, the central part is loosely arranged, forming no Regular voids, so-called alveolar structures.
(3) Embryonic rhabdomyosarcoma: The tumor cells are mostly immature rhabdomyoblasts and primitive mesenchymal cells in the early stage of embryonic development. The mucosa is present in the tumor. The cut surface is mucous edema, the cytoplasm is dark red, and the nucleus is darkly stained. Figure 1 A, B), the intersection of tumor cell types is sometimes seen in the above classification.
Prevention
Primary cardiac rhabdomyosarcoma prevention
1. Prevention of tumors is different from the prevention of common infectious diseases. Because its etiology and pathogenesis are complex, its meaning is far from being able to include only the elimination of pathogenic factors. Therefore, the concept of cancer prevention highlights the elimination of the cause, that is, the prevalence of cancer. On the basis of etiology, reduce or eliminate pathogenic factors, treat precancerous conditions, and fully use comprehensive treatment to improve the treatment level of tumors, reduce the mortality rate, and maximize the effective control of tumors.
2. Early detection, comprehensive diagnosis and early treatment of comprehensive measures, the disease is the first choice for surgical resection, but can be completely removed, although partial resection can alleviate clinical symptoms, but the prognosis is poor, the disease is not sensitive to radiotherapy, chemotherapy.
Complication
Primary cardiac rhabdomyosarcoma complications Complications, congestive heart failure, pericardial effusion, syncope, arrhythmia
Congestive heart failure, pericardial effusion, syncope, arrhythmia and other complications may occur.
Symptom
Symptoms of primary cardiac rhabdomyosarcoma Common symptoms Dyspnea, hepatosplenomegaly, pulmonary embolism, pleural effusion, syncope
The age of onset is mostly 30 to 40 years old. The incidence rate of males and females is roughly equal. The general medical history is short, and the clinical manifestations are mainly based on the location of the tumor and the obstruction of the heart.
Early patients often have fever, anorexia, general malaise, weight loss and other symptoms of malignant diseases. When the tumor develops to a certain extent, it can cause heart murmur, chest pain, pleural effusion, dyspnea and embolism of various organs. Among them, cardiopulmonary embolism and cerebral embolism are the most common.
When the tumor affects cardiac output, symptoms such as shortness of breath, chest pain, and congestive heart failure occur, typically manifesting as progressive unexplained congestive heart failure, especially right heart failure, pericardial effusion, syncope, Arrhythmia, vena cava obstruction and sudden death, often accompanied by pericardial effusion and pleural effusion, due to more common in right atrial rhabdomyosarcoma, its symptoms and signs are often confused with right atrial myxoma, the main clinical manifestations are Right heart failure, the vena cava pressure is blocked due to obstruction of peripheral blood flow, obstruction, upper and lower extremity edema and hepatosplenomegaly may occur, due to rapid growth of sarcoma and infiltration into the heart muscle, obstruction of cardiac blood flow or distant metastasis and death From the onset of symptoms to death for weeks to 2 years, according to the literature, about 75% of cardiac sarcomas have distant metastases, common metastases are lung, chest lymph nodes, mediastinum and rectum, followed by liver, kidney, adrenal gland, bone Wait.
Examine
Examination of primary cardiac rhabdomyosarcoma
The immunochemical method of myosin can identify rhabdomyosarcoma.
1. Radionuclide gate pool scanning is used to diagnose tumors in the chamber and chamber myocardium. This type of examination has low sensitivity to small filling defects, so it has lower resolution than ultrasound imaging and cardiovascular imaging, but when some other examinations When the ventricular and myocardial tumors are unclear, the radionuclide scan provides a clearer image of the filling defect.
2. Electron computed tomography (CT) CT can clearly show the heart chamber, muscle wall, pericardium and its relationship with surrounding structures, and is a non-invasive method for the diagnosis of cardiac tumors and differential diagnosis with paraventricular masses, but only For cross-sectional scanning, contrast agents need to be injected, and the contrast resolution is far less than MRI.
3. Magnetic Resonance Imaging (MRI) It can display the contours of cardiac tumors. In some cases, it can show the size, shape and features of the tumor surface, and the image is clearer than two-dimensional echocardiography and CT. The contrast agent is injected, but the cost of the examination is relatively high.
4. Cardioangiography Although cardiac catheterization and selective pulmonary angiography are not necessary for all cardiac tumors, most cases can obtain sufficient preoperative diagnosis information through two-dimensional ultrasound, CT, MRI. In some cases, more accurate information can be obtained through cardiac catheterization, although it is risky, but it is also necessary: 1 non-invasive examination can not determine the location or attachment of the tumor; 2 non-invasive examination Can not fully display the four-chamber view, and clinical and suspected multiple tumors; 3 other heart damage and tumor coexist may require different methods of surgery.
5. X-ray chest radiograph can show mediastinal, lung metastasis lesions, chest X-ray film common diffuse heart shadow.
6. Electrocardiograms usually show non-specific ST-T changes, but low voltage and bundle branch block can also occur.
Diagnosis
Diagnosis and diagnosis of primary cardiac rhabdomyosarcoma
diagnosis
For the unexplained progressive right heart failure, in addition to the conventional electrocardiogram, chest X-ray, echocardiography is essential, right heart angiography is also necessary, chest MRI should also be considered, but the final diagnosis still needs According to histology, the characteristics of the microscope, the arrangement of tumor cells is dispersed, the collagen fibers are few, and the tumor cells and tumor tissues are pleomorphic (small immature cells, binuclear, multinuclear giant cells, characterized by myogenic cells, such as cells). Rich in pulp, irritating red).
Differential diagnosis
Identification of diseases such as cardiac myxoma, metastatic heart tumor, and hyperthyroidism.
1. Cardiac myxoma Myxoma is benign, from the atrial septum, often pedicled, more common in the left atrium, its clinical manifestations are very similar to mitral stenosis, characterized by tumor obstruction of the mitral valve in the sitting position, the symptoms are dyspnea, Blemishes, fainting, pulmonary edema, obstruction in the supine position to eliminate symptoms, patients may have left atrial enlargement, apical area thunder-like diastolic murmur, but no open flap sound, right atrial myxoma clinical manifestations similar to tricuspid stenosis, Cardioangiography is helpful for diagnosis.
2. Metastatic cardiac tumors are more common than primary cardiac tumors, most of which are directly spread by adjacent lymphoid tissues. The common primary tumors are bronchial lung cancer and breast cancer. The metastatic sites are mainly atrial and pericardium, endocardium and heart. The valve is less involved, the right side is more common than the left side. The clinical manifestations are heart failure, arrhythmia and pericardial effusion. When the pericardium is involved, a large amount of pericardial effusion can be produced, resulting in cardiac tamponade. The puncture fluid is bloody and can be found. Cancer cells, myocardial invasion is prone to arrhythmia, X-ray examination showed limited or blurred shape of the heart shadow, pericardial effusion, weakened heart beats help diagnosis.
3. Hyperthyroidism is more common in women, 20 to 40 years old, clinical manifestations are diverse, cardiovascular system patients complain of palpitations, chest tightness, shortness of breath during exercise, pulse acceleration, severe arrhythmia, heart enlargement, heart failure, etc., basal metabolism Rate determination and radionuclide examination are helpful in the diagnosis of this disease.
